Carney triad is a rare condition that describes
the occurrence of three kinds of endocrine tumors in the same patient.
The tumors comprising the triad are tumors
in the gastrointestinal tract (known as gastrointestinal stromal tumors, or GIST),
pulmonary chondromas, and paragangliomas. These masses grow chiefly in the stomach,
the lungs, or the neuroendocrine tissues of the head, neck, and torso. Carney
triad is extremely rare and is most common in females.
Children with Carney triad are treated at Dana-Farber/Boston
Children's through our Endocrine-Oncology Program. Advanced cancers may
also be treated through our Solid
Tumor Center. Our integrated pediatric oncology service offers—in
one specialized program—the combined expertise of a leading cancer center and a
premier children’s hospital. We build a team to treat your child consisting of
oncologists, endocrinologists, genetic counselors, and surgeons.
As a parent,
you undoubtedly want to know what may have caused your child’s condition. Carney triad is likely related to genetic factors,
but the exact cause is unknown.
The symptoms of Carney triad may vary from child to child
and depend on where tumors are located and what kind they are. Symptoms might
mimic other, more common ailments. Some symptoms may include:
- Gastrointestinal bleeding
- Stomach pain
- Abdominal mass that can be felt on examination
- Anemia (low red blood cell count)
- Headaches
- High blood pressure
Because many of these symptoms can also point to
other conditions, it’s important to have your child evaluated by a qualified
medical professional right away.
The first step in treating your child is forming an
accurate and complete diagnosis. Your child’s physician may order a number of
different tests including:
- A
physical exam and complete medical history.
- Blood
and urine tests.
- Endoscopy,
a minor procedure in which a flexible camera is inserted through the mouth and
into the stomach.
- A
biopsy, a tissue sample taken from the tumor. The tumor's appearance under a
microscope helps doctors to make a diagnosis so the appropriate treatments can
be recommended.
- Magnetic
resonance imaging (MRI), a diagnostic
procedure that produces detailed images of the area where the tumor is located.
An MRI uses a combination of large magnets, radiofrequencies, and a computer to
analyze organs and structures within the body.
- A
computerized tomography scan (CT/CAT scan), an imaging technique that provides
more detailed pictures than X-rays.
- Molecular
testing to determine whether the tumor is linked to specific genes.
There may be other
diagnostic tests that your doctor will discuss with you depending on your
child's individual situation. After we complete all necessary tests, our
experts meet to review and discuss what they have learned about your child's
condition. Then, we will meet with you and your family to discuss the results
and outline the best possible treatment options.
Treatment for
your child's Carney triad will depend on a number of factors, including the
type of tumors your child has and where they are located. Your child's doctor
may recommend:
- Surgery, involving biopsy and removal
of the entire tumor and nearby tissue.
- Radiation,
the use of high-energy rays from a specialized machine to damage or kill cancer
cells and shrink tumors. This is often used together with surgery, either
before or after removal of the tumor.
- Chemotherapy, a drug treatment
that aims to destroy or shrink cancer cells, may be given before or after
surgery.
- Different groups
of chemotherapy drugs work in different ways. Your child may receive chemotherapy
orally, as a pill to swallow; intramuscularly, as an injection into the muscle
or fat tissue; intravenously, as a direct injection into the bloodstream or IV;
or intrathecally, as a direct injection into the spinal column through a
needle. Often, a combination of chemotherapy drugs is used.
- While
chemotherapy can be quite effective in treating certain cancers, the drugs
cannot differentiate normal healthy cells from cancer cells. As a result, there
can be adverse side effects during treatment. Being able to anticipate these
side effects can help the care team, child, and family prepare and, in some
cases, prevent these complications from occurring, if at all possible.
Children who are treated through our Endocrine-Oncology Program benefit from
the work of our basic and clinical researchers, who are striving to understand
the scientific causes of endocrine cancers. Their work can result in the
introduction of new treatment options. We are a world leader in translational
research, bringing laboratory advances to the bedside and into doctors’ offices
as quickly as possible.
Clinical Trials
Clinical
trials, or research studies evaluating new treatment approaches, are a major
offering at Dana-Farber/Boston Children’s. For many children with rare or
hard-to-treat conditions, clinical trials provide new options.
It’s possible
that your child will be eligible to participate in one of our clinical trials.
In addition to launching our own clinical trials, we also offer trials
available through collaborative groups such as the Children's Oncology Group (COG).
If your child has a progressive or recurrent
tumor, she may be eligible for a number of experimental therapies available
through these groups or from one of our independent clinical investigators.
Children treated for Carney triad should visit a
survivorship clinic yearly. Through the David
B. Perini, Jr. Quality of Life Clinic, our cancer survivorship
clinic, childhood cancer survivors receive a comprehensive follow-up evaluation
from their cancer care team. In addition to meeting with your pediatric
oncologists, your child may see one of our endocrinologists, cardiologists, neurologists,
neuro-psychologists, or alternative/complementary therapy specialists. We also offer patient and family
education, psychosocial assessment, genetic counseling, reproductive counseling, and opportunities to speak with other
childhood cancer survivors.